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Unexplained anaemia an unusual case report Unexplained anaemia an unusual case report

Unexplained anaemia an unusual case report - PowerPoint Presentation

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Unexplained anaemia an unusual case report - PPT Presentation

Department of Radiology Queen Alexandra Hospital Portsmouth Hospitals NHS Trust 18th BSGAR Annual Meeting 35 February 2016 Birmingham Authors Dr A Higginson Consultant GI Radiologist ID: 914313

nhs amyloidosis amyloid calcifications amyloidosis nhs calcifications amyloid porthosp omentum follow diffuse doi amp consultant peritoneal deposition conditions anaemia

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Slide1

Unexplained anaemiaan unusual case report

Department of RadiologyQueen Alexandra HospitalPortsmouth Hospitals NHS Trust

18th

BSGAR Annual

Meeting

3-5

February

2016

Birmingham

Slide2

AuthorsDr A. Higginson, Consultant GI RadiologistEmail: Antony.Higginson@porthosp.nhs.uk

Dr R. Beable, Consultant GI Radiologist Email: Richard.Beable@porthosp.nhs.uk

Dr A. Yazdi,

Consultant GI

Radiologist

Email:

Amir.Yazdi@porthosp.nhs.uk

Slide3

Clinical presentation59 year old female

Presents with fatigue, anaemia, weight loss and ? hematemesis.LAB: Routine: unremarkable.Ferritin: 588 (12-250).OGD: Vascular ectasia, patchy erythema → ? gastric antral

vascular

ectasia

.

GAVE-Syndrome (watermelon stomach):

Rare, unknown pathogenesis.

Anaemia, GI bleed and abdominal pain.

Associated with variety of conditions (hepatic, renal, cardiac).

Resembles portal hypertensive

gastropathy

.

Slide4

OGDWill follow

Slide5

CTWill follow

Slide6

CT findingsDiffuse nodular infiltration of omentum

with calcifications.Retroperitoneal deposit (left ureter).No ascites.Small pulmonary nodules.

Slide7

Additional clueSerum

free lambda chain: 119 (6-26)

Slide8

DiagnosisPathology

(16G core omentum):Widespread eosinophilic deposition in vessel walls & interstitium.Diffuse histiocytic

infiltrates.

Focal calcifications & ossification.

Congo red stain + birefringence: strongly positive.

→ characteristic of

amyloidosis

.

US

image Will follow

Slide9

Discussion

Amyloidosis:Systemic vs. localized deposition of amyloid.Primary: associated with myeloma, lymphoma.Secondary: associated with RA, TB, Crohn.GI (most commonly), but also GU, cardiovascular,

msk

and CNS.

GI:

Gastro-oesophageal:

dysmotility

, reflux, wall thickening (amyloid in

muscularis

,

Auerbach

plexus)

Small bowel: diffuse or nodular wall thickening.

Splenomegaly, with ↑ risk of spontaneous rupture.

Hepatomegaly, with ↓ attenuation (amyloid deposition).

Peritoneum &

Omentum

: diffuse nodules, +/- calcifications.

Can mimic disseminated malignancy, but has indolent clinical course.

Slide10

Take home messageClinical and imaging features of amyloidosis are non-specific and diverse.

Amyloidosis in DD. Unexplained weight loss, GI bleeding.History of chronic inflammatory disease, myeloma.Can mimic peritoneal carcinomatosis, but indolent course.

Slide11

References

Omental and Peritoneal Involvement in Systemic Amyloidosis: CT with Pathologic Correlation. Horger

et al. doi:10.2214/AJR.05.0638.

Unusual

Nonneoplastic

Peritoneal

and

Subperitoneal

Conditions: CT findings.

Pickhardt

et al.

doi

110.1148/rg.253045145.

Amyloidosis of the gastrointestinal tract: a 13-year,

single-

center

, referral experience.

Cowan et al. doi:10.3324/haematol.2012.068155.

Amyloidosis:

Review and

CT

Manifestations.

Georgiades

et al.

d

oi

10.1148/rg.242035114